Bilateral mandibular regional odontodysplasia with vascular nevus H. Robert Steiman, PhD, DDS, MS Claire L. Cullen, DMD James R. Geist, DDS, MS

Abstract Odontodysplasiais a rare clinicopathologic condition that has a variety of expressions, including a range of tooth coloration, formation of hypoplastic enamelaffecting one or multiple teeth, bizarre radiographic appearance,and delayed eruption of teeth. An unusualcase is presented of bilateral regional odontodysplasia associated with a vascular nevus of the face and neck. A review of the etiology with emphasison the "local circulatory disease" theory is presented. (Pediatr Dent 13:303-6, 1991)

Introduction Odontodysplasia is a clinicopathologic disorder that fected area occurred during the time of tooth develop- affects developing dental follicles (Lian et al. 1988). The ment (Gibbard et al. 1973; Fearne et al. 1986). This reader is directed to a review by Crawford and Aldred disturbance took the form of a vascular nevus or "nevus (1989). The condition was first described as a case flammeus," which is a congenital vascular lesion that unerupted primary teeth and later referred to as ar- disappears gradually (Walton et alo 1978). Clinically, rested tooth development, odontogenesis imperfecta, presents as pale pink in color and is also knownas a ghost teeth, and Rushton’s shell teeth. The term "salmon patch" (Walton et al. 1978). The vascular theory odontodysplasia has been used since 1963, with re- is debatable since proof is not evident in every patient gional or local as a further descriptive since 1970 (Lustmannet al. 1975). (Zegarelli et al. 1963; Pindborg1970). There are a variety Odontodysplasia is considered an uncommonden- of expressions. Local or regional distribution in more tal finding (Gibbard et al. 1973). Approximately 108 than one quadrant has been noted, with most affected cases have been reported in the world literature teeth found in the maxillary anterior region (Crawford (Crawford and Aldred 1989). This report presents and Aldred 1989). Both primary and permanent denti- unusual case of RODin the mandible, which crossed tions may be involved (Crawford and Aldred 1989). the midline and was accompanied by a congenital vas- The affected teeth appear malformed with hypoplastic cular nevus. areas, discolored from yellow to a brown opalescence. Arrested root formation is common,and manyteeth fail Case Report to erupt (Gibbard et al. 1973). These underdeveloped An l 1-year-old Caucasian .boy was seen by an teeth appear indistinct or ghost-like on radiographs endodontist to evaluate swelling in the labial fold adja- because of the hypoplastic enamel and dentin (Crawford cent to the mandibular left permanent central incisor. and Aldred 1989). The tooth was diagnosed as necrotic, and endodontic The presenting symptomsinclude failure or delay of treatment was initiated. The patient was referred to the eruption of teeth, premature exfoliation or extraction of University of Detroit School of Dentistry with a further teeth, abscess formation due to pulp exposures in hypo- complaint of missing and malformed teeth. plastic surfaces, malformed teeth, and noninflammatory The patient was a product of a normal pregnancy jaw swelling, which presents as a firm, painless, soft- and delivery. At birth, he displayed two large, reddish, tissue enlargement of the gingiva around the affected fiat congenital vascular anomalies located under his teeth (Dayal and Mani 1981; Neupert and Wright 1989). chin and on the left side of his face below the ear. These The etiology of regional odontodysplasia (ROD) lesions faded with age; however, fiat, reticulated, red- unknown. However, numerous theories have been sug- dish nevi were present in the aforementioned areas on gested, including local circulatory disorders, viral in- examination (Fig 1, see next page). The medical history fections, prenatal drug exposure, neural crest cell de- revealed he had been hospitalized at one year of age fects, trauma, and infection (Crawford and Aldred 1989). with a fever and swelling of the left side of his face; this Currently, the most commonlyaccepted theory is vas- resolved following antibiotic therapy. The etiology was cular etiology, since many cases have shown that a never determined. At the time of examination, he was disturbance in the local circulation overlying the af- in good health. No other memberof his family had any

PEDIATRIC DENTISTRY: SEPTEMBER/OCTOBER,1991 - VOLUME13, NUMBER5 303 Fig 1: Vascular nevi presented in soft tissue overlying the Fig 3: Hypoplastic and discolored permanent and primary teeth. affected area. Arrows indicate outline of lesions.

surrounding the unerupted canines were enlarged, and small radiopaque foci representing dystrophic calcifi- cation were identified in the follicles surrounding the partially erupted lateral incisor (Fig 4). Discussion Epidemiologic data derived from more than 100 re- ported cases indicate that regional odontodysplasia is 2.5 times more common in the maxilla (Crawford and Aldred 1989). However, our patient pre- Fig 2: Bilateral manifestation of ROD. sented with a mandibular le- dental abnormalities. sion. When more The intraoral clinical and radiographic examinations than one quad- revealed a structurally normal, age-appropriate maxil- rant is involved, lary permanent dentition with severe crowding of the ROD can be seen incisors. In the mandibular arch, the right posterior on the same side segment and the four permanent incisors were visible of both jaws or clinically, while the dentition in the entire permanent bilaterally in ei- left posterior quadrant was unerupted. The dysplastic ther the maxilla permanent left first molar and the primary left first and or mandible, second molars had been extracted in response to epi- as in this patient sodes of noninflammatory swelling of the gingivae (Crawford and around the affected teeth. An orthodontic appliance Aldred 1989). was present in the mandibular right quadrant, and a Our case is temporary restoration was noted in the access opening somewhat of the mandibular left central incisor (Fig 2). unusual, how- Clinically, ROD affected both the primary and per- ever, in its manent dentitions. The permanent and primary pattern of bi- anteriors were yellow with hypoplastic enamel (Fig 3). lateral distribu- Radiographically, the unerupted teeth appeared un- tion (Lustmann Fig 4: Radiodensity of affected teeth was 1975 found that reduced as compared to normal teeth; derdeveloped with large pulp chambers and little de- note large pulp chambers, large follicles marcation between the enamel and dentin. The affected teeth around the canines, and small radiopaque radiodensity of these teeth was reduced in comparison were located on foci in the follicles of the partially erupted to the unaffected mandibular teeth. The dental follicles both sides of the lateral incisor.

304 PEDIATRIC DENTISTRY: SEPTEMBER/OCTOBER , 1991 ~ VOLUME 13, NUMBER 5 midline in only 11 of the 51 cases reported to that time). Numerous theories on the etiology of RODhave Normal teeth generally are not found mesial to the been proposed, but compelling evidence is lacking for affected dentition (Gibbard et al. 1973). In addition, most of them. Rushton (1965) investigated viral infec- RODis usually seen in consecutive teeth, with no nor- tions as the possible cause of ROD. However, other mal teeth interposed (Crawford and Aldred 1989). The authors have been unable to detect them, nor do the present case is consistent in these respects. It also has enamel defects in RODresemble defects caused by been reported that the permanentfirst molar is dysplas- known viruses such as rubella or cytomegalovirus tic only if the adjacent primary molars exhibit ROD (Crawford and Aldred 1989). Likewise, no specific group (Gibbard et al. 1973). The mandibular right permanent of prenatal drug exposures can be correlated to the first molar and primary second molar were both normal defect (Crawford and Aldred 1989). Neural crest cells in this patient. Onthe left side, however, the mandibu- that fail to migrate are being investigated as a possible lar left permanent first molar and primary second mo- cause (Crawford and Aldred 1989). Trauma to the den- lar had been extracted, possibly because they were both tition has not been well supported as a possible cause; affected with ROD. however, several investigators have reported it The patient’s presenting complaints of missing and (Anneroth and Ramstrom 1980). Walton and associates abnormal teeth are typical of this condition. Delayed (1978) suggested that local vascular disturbances may eruption of the permanent dentition is common,as is be involved in ROD.They reported three RODpatients extraction of dysplastic teeth as a result of abscesses in with congenital cutaneous angiomas, and drew atten- the absence of caries (Pruhs et al. 1975; Anneroth and tion to three previously reported cases with similar Ramstrom1980). The unusual clinical and radiographic vascular lesions. Our patient exhibited remnants of appearance of the teeth can be related to defective vascular birthmarks on the skin overlying the left side production of enamel and dentin. The enamel is hyp- of the mandible. Although the cutaneous lesions them- oplastic, and the presence of residual enamel matrix selves are not likely to cause alterations in tooth forma- indicates incomplete mineralization of the matrix tion, they may be a marker of an underlying vascular (Crawford and Aldred 1989). A differential diagnosis disturbance that could affect odontogenesis, as dis- may include amelogenesis imperfecta, dentinogenesis cussed by Fearne et al. (1986). The fact that both primary imperfecta, dentinal dysplasia I and II, shell teeth, and permanent teeth are affected in RODleads to the hypophosphatasia, rickets, Turner’s tooth, nursing car- conclusion that the etiologic agent must be present from ies, and calcifying odontogenic fibroma (Crawford and the second trimester of gestation, when calcification Aldred 1989). begins in the primary dentition, through the early teen- A microradiographic examination of RODby Kerebel age years during calcification of the permanent teeth. et al. (1989) demonstrated hypomineralized incre- Therefore, if a cutaneous angioma is related to alter- mental strands of tissue interspersed throughout nor- ation in regional blood supply to the teeth over a suffi- mal layers of enamel. This indicates that amelogenesis cient period of time, this theory could be plausible. is not only defective, but actually is interrupted as the Unfortunately, vascular lesions are not identified in all tooth forms. Some layers of enamel are actually well patients. mineralized (Kerebel and Kerebel 1982; Fearne et al. Another proposed factor in the pathogenesis of ROD 1986), but this does not affect the radiographic image; is local or systemic infection (Zegarelli et al. 1963; the severe hypoplasia imparts a subnormal radiopacity Rushton 1965; Fearne et al. 1986). Our patient’s history to the enamel. The dentinal layer is similarly hypoplas- of fever and swelling of the left side of the face in tic, and dentinal tubules are reduced in number and infancy is intriguing since this was the side with the irregular in arrangement, although the mantle dentin affected teeth. However,if infection were to result in usually is normal (Kinirons et al. 1988). Interglobular damage to primary teeth, it would have to affect the dentin and poor calcospherite formation are detected in den~al lamina in utero. Furthermore, attempts to iden- varying degrees (Crawford and Aldred 1989). Histo- tify viral particles or antibodies in RODpatients have logic examination of dentin has revealed the presence been fruitless (Gibbard et al. 1973). of "clefts" or widened tubules extending through the mantle dentin to the pulp (Abrams and Groper 1966). Summary is the hypoplasia and hypocalcification of hard tissue, RODwith an unusual presentation of bilateral man- combined with the large pulp chambers (Lian et al. dibular distribution accompanied by a cutaneous vas- 1988), that accounts for the "ghost-like" radiographic cular lesion is presented. Although this is another case appearance of ROD. Clefts in enamel and dentin may of RODcoexisting with vascular lesions, the causal allow entry of microorganisms into the pulp, causing relationship remains uncertain. pulpitis and periapical inflammation without caries.

PEDIATRIC DENTISTRY: SEPTEMBER/OCTOBER,1991 ~ VOLUME13, NUMBER5 305 Dr. Steiman is professor and chairman, Departmentof Endodontics, Kinirons MJ, O’Brien FV, Gregg TA: Regional odontodysplasia: an Schoolof Dentistry, University of Detroit, Detroit, MI.Dr. Cullenis evaluation of three cases basedon clinical, microradiographicand associate professor, Faculty of Dentistry, University of WesternAus- histopathologicalfindings. Br Dent J 165:136-39,1988. tralia, Perth, WesternAustralia. Dr. Geist is associate professor and Lian CB, ChongBS, Siar CH,Phang YC: Ghost teeth. Casereport. Aust interim chairman, Departmentof Stomatology,School of Dentistry, DentJ 33:291-94,1988. Universityof Detroit, Detroit, MI. LustmannJ, Klein H, UlmanskyM. Odontodysplasia. Report of two cases and reviewof the literature. Oral Surg39:781-93, 1975. AbramsAM, Groper J: Odontodysplasia.Report of three cases. J Dent Neupert EA, Wright JM: Regional odontodysplasia presenting as a Child 33:353~;2,1966. soft tissue swelling. Oral Surg67:193-96, 1989. Anneroth G, RamstromG. Unilateral odontodysplasia. Swed Dent J Pindborg JJ: Pathology of the Dental Hard Tissues. Copenhagen: 4:93-100, 1980. Munksgaard,1970, pp 120-23. Crawford PJM, Aldred MJ: Regional odontodysplasia: a bibliogra- Pruhs RJ, SimonsenCR, SharmaPS, Fodor B: Odontodysplasia. J Am phy. J Oral Pathol Med18:251-63, 1989. Dent Assoc 91:1057-66,1975. Dayal PK, Mani NJ: Odontodysplasia, report of a case. l Oral Med RushtonMA: Odontodysplasia: "ghost teeth’. Br Dent J 119:109-13, 36:79M~1,1981. 1965. Fearne J, Williams DM,Brook AH: Regional odontodysplasia: a Walton JL, Witkop CJ Jr, Walker PO: Odontodysplasia. Report of clinical and histological evaluation. J Int AssocDent Child 17:21- three cases with vascular nevi overlying the adjacent skin of the 5, 1986. face. Oral Surg46:676-84, 1978. GibbardPD, Lee KW,Winter GB:Odontodysplasia. Br Dent J 135:525- Zegarelli EV, Kutscher AH, Applebaum E, Archard HO: 32, 1973. Odontodysplasia.Oral Surg 16: 187-93, 1963. KerebelB, KerebelLM: Structural, ultrastructural, micro-radiographic, and electron-probe studies of an unusual case of regional odontodysplasia. J Dent Res 61:1056-62,1982.

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